[Multicystic presentation of a lepidic mucinous adenocarcinoma]. / Présentation multi-kystique d'un adénocarcinome lépidique mucineux.

INTRODUCTION: The pneumonic form is very characteristic of lepidic pattern adenocarcinoma of the lung. However, the diagnosis and treatment of the disease when it presents in this way may be delayed by atypical radiological findings and severity of hypoxemia. CASE REPORT: A 48-year-old female, non-smoker, asthmatic since the age of 20 years, was hospitalized for a diffuse infiltrative pneumonia complicated by severe respiratory failure. The history included chronic cough, gradually increasing dyspnoea on exertion lasting for 14 months and the onset of haemoptysis of low abundance associated with arthralgia in the previous month. She had no professional or domestic exposure to any risk factors. Chest CT scan revealed bilateral alveolar condensations, ground glass areas with thickened septa creating a crazy paving pattern, and numerous large cysts of various sizes and locations, often with irregular thin walls. Microbiological and immunological tests were negative. She required early invasive ventilation and then venovenous ECMO together with broad-spectrum empiric antibiotic therapy, but died after 39 days in intensive care. The autopsy revealed lesions consistent with mucinous lepedic adenocarcinoma with no EGFR mutation and KRAS mutation. There was also associated pulmonary suppuration. CONCLUSION: Mucinous lepidic adenocarcinoma is an alternative diagnosis for pneumonic consolidation associated with multiple cysts.

[AA-type amyloidosis secondary to multidrug resistant pulmonary tuberculosis: implications for therapy]. / Amylose AA secondaire à une tuberculose pulmonaire multi-résistante: implications thérapeutiques.

Multidrug resistant pulmonary tuberculosis was diagnosed to a 32-year-old man. An AA-amyloidosis was subsequently diagnosed on the renal biopsy performed for nephrotic syndrome and macroscopic hematuria. A 6-drug antibiotic treatment was delivered quickly after first results of genotypic antibiogram given the renal failure, and was secondarily adapted to the phenotypic antibiogram. Multidrug therapy was fairly well tolerated. Clinical and biological improving were slow. Although tuberculosis is a classic cause of amyloidosis, this is the first case reporting an association between a multidrug resistant case and an amyloidosis in adults. This case also raises the question of MDR probabilistic treatments in situations whether a vital organ prognosis is engaged.

Comparing non-enhanced and enhanced sequences in the assessment of effusion and synovitis in knee OA: associations with clinical, macroscopic and microscopic features.

OBJECTIVE: The purpose of this study was to evaluate synovial membrane (SM) inflammation and joint effusion scores by semiquantitative magnetic resonance imaging (MRI) assessment with and without enhanced sequences. Gold standards used for comparison were microscopic examination of SM biopsies for SM inflammation and joint volume measurement (JVM) after arthrocentesis for effusion. METHODS: Patients (n = 30) fulfilling ACR criteria for knee osteoarthritis (OA) and requiring joint lavage, were evaluated with MRI: (1) SM inflammation was assessed by Whole-Organ Magnetic Resonance Imaging Score (WORMS) on T2 weighted sequences (T2w) a composite score assessing together synovitis and effusion, and the MRI-synovitis score (based on synovitis thickening in five regions of interest) on a T1-weighted fat sat sequence after contrast agent injection (T1wCE). (2) Joint effusion was evaluated by MRI-effusion score (T1wCE) and the WORMS (T2w). JVM was measured after arthrocentesis, and microscopic SM inflammation was determined in SM samples (n = 86). Correlations between MRI scores and clinical, biologic and histologic parameters were studied. RESULTS: Both scores for effusion were well correlated [r = 0.82 (0.65-0.91); P < 0.001] and presented excellent intraclass correlation coefficient (ICC) for intra- and inter-observer reproducibility. MRI scores for effusion were well correlated with JVM (r = 0.60 for WORMS and r = 0.59 for MRI-effusion score). Synovitis scores were highly reproducible but moderately correlated (r = 0.63; P < 0.001). Only MRI-synovitis total score (T1wCE) was correlated with SM microscopic inflammation (r = 0.46; P = 0.01) and most strongly infiltration (r = 0.45; P < 0.005). CONCLUSIONS: T2w sequences are adequate in assessing effusion volume in compare to joint volume by arthrocentesis but only T1wCE sequences are able to detect synovitis according to the reference of synovial biopsy.

Magnetic resonance imaging in osteoarthritis: which method best reflects synovial membrane inflammation? Correlations with clinical, macroscopic and microscopic features.

OBJECTIVES: : To study synovial membrane (SM) inflammation near the patella with different magnetic resonance imaging (MRI) approaches performed using a T1-injected sequence in knee osteoarthritis (OA), and to compare MRI results with macroscopic, microscopic and clinical findings. METHODS: Fifteen patients fulfilling American College of Rheumatology (ACR) criteria for knee OA and requiring joint lavage completed a functional index (Lequesne's functional index) and a pain visual analog scale (VAS). SM inflammation near the patella was assessed on axial fat saturation post-injected T1 MRI images using three different methods: (1) semi-quantitative score=MRI synovitis score; (2) synovial membrane volume (SMV) analysis; (3) SMV with low (SMVL) (<0.3%/s(-1)), intermediate (SMVI) (0.3%/s(-1) to 1%/s(-1)) and high (SMVH) (> or =1%/s(-1)) speed of enhancement. Chondral lesions and SM inflammation were macroscopically graded and SM biopsies performed for microscopic scoring. RESULTS: All MRI approaches exhibited excellent intra- and inter-observer reproducibility. MRI synovitis score correlated well with macroscopic (r=0.61, P=0.003) and total microscopic scores (r=0.55, P=0.03). Correlations between SMV and macroscopic (r=0.60, P=0.02) and microscopic congestion (r=0.63, P=0.01) were good. SMVH was correlated only with microscopic congestion (r=0.79, P=0.01). Low SMV was associated with neither macroscopic nor microscopic scores. However, it did correlate well with pain-VAS score (r=0.61, P=0.03) and moderately with a functional index (r=0.46, P=0.10). CONCLUSION: The three MRI approaches used here provided highly reproducible information on SM inflammation near the patella in knee OA. Compared to SMV, MRI synovitis score seems sufficient to assess synovial inflammation but high SMV is an appropriate indicator of vascular congestion, and low SMV reflects pain in knee OA.

Molecular diagnosis of disseminated adiaspiromycosis due to Emmonsia crescens.

Emmonsia crescens is a saprophytic fungus that is distributed worldwide, causing diseases mostly in rodents. It has also been described, though rarely, as an etiologic agent of pulmonary pathology in humans, potentially leading to death. A case of pulmonary adiaspiromycosis is reported in a 30-year-old immunocompetent man. The patient presented with a history of several weeks of weakness, cough, fever, and weight loss of 10 kg. Clinical and radiographic findings showed pulmonary lesions consistent with tuberculosis or histoplasmosis, but no pathogen was found with classical microbiological procedures. The diagnosis of adiaspiromycosis due to Emmonsia crescens was initially made using molecular biology techniques. Histological observations subsequently confirmed the presence of adiaspores in granulomas. To our knowledge, this is the first case of adiaspiromycosis diagnosed by PCR and sequencing. The patient was treated with itraconazole and was seen at 1 month with symptomatic improvement. Here we will discuss this rare fungal infection and its difficult treatment and diagnosis. As represented in this case, molecular biology is a powerful method to optimize diagnostic tests and therefore improve the care of the infected patient.

[Imaging of serious visceral forms of mucormycosis in five immunodepressed patients]. / Imagerie des formes viscérales graves de la mucormycose chez 5 patients immunodéprimés.

OBJECTIVES: Show the contribution of imaging to the diagnosis and follow-up of serious mucormycosis in immunodepressed patients. MATERIALS AND METHODS: Retrospective analysis of the 5-year radiological data in serious forms of mucormycosis occurring in patients with bone marrow allografts who are in refractory chronic graft-versus-host disease after bone marrow transplantation from 2002 to 2005. The positive diagnosis was bacteriologically and pathologically positive in all cases. RESULTS: This study involved three patients with isolated pulmonary involvement and two cases of disseminated mucormycosis. Areas of pulmonary condensations were found in all cases, one of whom had a low-attenuation zone and parenchymal nodules. The kidney, liver, and spleen lesions were clearly limited, hypoechogenic, hypodense, and homogenous with no peripheral contrast material uptake. There was thyroid involvement in the form of hypoechogenic nodules. Rapid growth of the lesions was observed on follow-up CT (n=3) and despite surgical treatment (n=2) and intensive medical management, all cases ended in death. CONCLUSION: Mucormycosis is an integral part of the differential diagnosis of infectious syndromes in immunodepressed patients during the period after bone marrow transplantation. Imaging can assist in the diagnosis but pathological confirmation remains indispensable.

[Palpebral edema secondary to treatment by a specific inhibitor of tyrosine kinase: Glivec. A case report]. / OEdème palpébral secondaire au traitement par inhibiteur de la tyrosine kinase: Glivec. A propos d'une observation anatomo-clinique.

Iatrogenic causes for palpebral edema are rarely suspected and must be specifically sought. The authors report a case of palpebral edema in a patient treated with a specific inhibitor of the BCR-ABL tyrosine kinase (Glivec) for chronic myeloid leukemia. Histopathological analysis of specimens of the excised upper eyelid tissue revealed the absence of leukemic infiltration, suggesting the toxic effect of the treatment. This side effect should be noted so that patients are informed and biopsy is proposed to eliminate tumoral infiltration.

Dynamic MR imaging of bladder haemangioma.

Haemangioma is a rare benign mesenchymal tumour of the bladder. We report a case of bladder haemangioma and describe the enhancement kinetics observed on MR images after gadolinium bolus administration, which is similar to that reported in liver haemangioma.

[Formaldehyde fixation in the third millennium]. / La fixation formolée à l'aube du troisième millénaire.

This review deals with the general principles and problems of formaldehyde fixation. After a short description of 1) formaldehyde methods of production, 2) chemical properties of formaldehyde solution, and 3) kinetic of formaldehyde binding in tissue, formaldehyde reactivity with the tissue biopolymers, proteins and cucleic acids mainly, are described. How formaldehyde fixation of tissues adversely affects the reactivity of cellular proteins with their respective specific antibody and the ways the most commonly used retrieval techniques in immunohistochemistry act are, thereafter, discussed. Finally, concerns that need to be dealt with when formalin-fixed specimens are used for genomic analysis and studies of DNA expression are highlighted.

Lymphoproliferative disorders after renal transplantation: role of medical imaging.

Post-transplantation lymphoproliferative disorders (PTLD) are a complication of immunosuppressed transplant recipients, and their incidence is reported to be 20-120 times greater than the rate in the general population. After kidney transplantation, PTLD more likely arise within the renal transplant fossa. Radiological patterns of these forms are presented and discussed, according to a review of the literature, and illustrated by cases from our institution. Ultrasound plays an essential role in the early diagnosis of PTLD by detecting a urinary obstruction associated with adenopathy or an ill-defined mass not previously seen. However, in the case of an inconclusive US examination, CT or MRI should be performed to confirm the presence of a mass. Both techniques are useful in evaluating the extension of the process within the transplantation fossa; MRI seems more accurate and can be used for the follow-up, especially after reduction in immunosuppressive therapy without transplant removal.

Amyloid and non-amyloid carpal tunnel syndrome in patients receiving chronic renal dialysis.

OBJECTIVE: To determine the prevalence of amyloid deposits among patients with carpal tunnel syndrome (CTS) receiving dialysis, and to investigate the factors associated with amyloid and non-amyloid CTS. METHODS: Subjects for this prospective study were dialysis patients who underwent surgery for CTS in the same surgical unit between 1989 and 1997. CTS was diagnosed from clinical and electromyographic (EMG) findings. Systematic standard radiographs and laboratory data were also obtained. Surgical investigations included systematic macroscopic examination and biopsy of the epineurium, flexor retinaculum, synovium, and flexor tendon sheaths. Samples were stained for amyloid and examined by plain and polarized light microscopy, immunohistochemistry, and electron microscopy. RESULTS: Forty-one samples from 30 patients (11 bilateral cases) were examined. Amyloid deposits were found in 26 samples from 18 patients (7 M, 11 F). Fifteen samples from 12 patients (3 M, 9 F) showed no amyloid deposits. Amyloid CTS was statistically significantly associated with arthralgia and longterm dialysis [mean 13.3 (range 5.5-23) vs 7.5 yrs (range 3 mo-14 yrs)] in non-amyloid CTS. Flexor tenosynovitis and carpal bone erosion occurred more frequently in amyloid CTS. There were no statistically significant differences between the 2 groups in clinical, laboratory or EMG findings, type of dialysis membrane, or frequency of ipsilateral fistula. Only amyloid CTS was recurrent. CONCLUSION: Amyloid deposits were confirmed microscopically in 63.4% of patients. The relatively large number of cases of non-amyloid CTS without signs of dialysis associated arthropathy suggests that CTS is not a satisfactory criterion for diagnosis of dialysis arthropathy or beta2-microglobulin amyloidosis unless the presence of amyloid has been confirmed or duration of dialysis treatment has been at least 15 years.

Clear cell rhabdomyosarcoma.

A clear cell rhabdomyosarcoma was studied by light microscopy, histochemistry, immunohistochemistry, and electron microscopy. It was a large, painful left parapharyngeal mass in a 10-year-old boy with intracranial extension and cervical metastatic enlarged lymph nodes. Tumor tissue was macroscopically grayish. At microscopic examination, the architecture was diffuse and focally alveolar. Tumor cells were of three types. Most cells were large, round or polygonal, with abundant clear vacuolated cytoplasm. Fibrils were sometimes found to be present around the nucleus. Nuclei often had irregular outlines and multiple nucleoli. Mitotic activity was high. Some round or elongated cells had eosinophilic fibrillar cytoplasm and were found to have a few double striations. A few cells were round and medium sized with a high nucleocytoplasmic ratio. Periodic acid-Schiff stain demonstrated huge amounts of intracytoplasmic glycogen in clear cells. Tumor cells showed positive immunostaining for muscle markers (desmin, muscle actins, dystrophin). Electron microscopy showed large lakes of glycogen, lipid droplets, and striated muscle features.

[Renal cell carcinomas in children. Apropos of two cases]. / Carcinomes à cellules rénales chez l'enfant. A propos de deux observations.

The authors report two cases of renal cell carcinoma in children. A tumor occurring in a eight-year-old girl was composed of granular and oncocytic cells with obvious nuclear atypia. The other renal neoplasm, occurring in a nine-year-old boy, had a papillary pattern and was composed of small cells with mild nuclear atypia. In both patients, the tumor was confined to the kidney; radical nephrectomy was performed and patients were free of disease-with a follow-up of four years and one year, respectively.